{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,2]],"date-time":"2026-01-02T17:17:45Z","timestamp":1767374265444,"version":"3.44.0"},"reference-count":13,"publisher":"BMJ","issue":"4","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2025,4,9]]},"published-print":{"date-parts":[[2025,4]]},"abstract":"<jats:p>Loeys-Dietz syndrome (LDS) is a rare connective tissue disorder that often leads to delayed wound healing due to mutations affecting the extracellular matrix. We describe a case of a man in his 20s with LDS type 4 presenting with a non-healing sternotomy wound following valve-sparing aortic root surgery. Initial management with simple dressings failed with non-healing over a 10-month period, necessitating scar revision surgery with layered closure and incisional negative pressure wound therapy (NPWT) for a prolonged period. This approach resulted in wound healing after 6\u2009months. Following a revisional valve replacement, a similar treatment led to healing within 2\u2009months. This case highlights the effectiveness of prolonged NPWT in managing complex wounds in patients with LDS, emphasising the need for specialised wound care in individuals with genetic predispositions to poor wound healing.<\/jats:p>","DOI":"10.1136\/bcr-2024-264200","type":"journal-article","created":{"date-parts":[[2025,4,22]],"date-time":"2025-04-22T15:20:22Z","timestamp":1745335222000},"page":"e264200","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["Management of non-healing sternotomy wounds in Loeys-Dietz syndrome"],"prefix":"10.1136","volume":"18","author":[{"given":"Justin","family":"Ho","sequence":"first","affiliation":[{"name":"Plastic and Reconstructive Surgery, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vithum","family":"Weerasekera","sequence":"additional","affiliation":[{"name":"Plastic and Reconstructive Surgery, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6060-1487","authenticated-orcid":false,"given":"Kai Yuen","family":"Wong","sequence":"additional","affiliation":[{"name":"Plastic and Reconstructive Surgery, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2025,4,22]]},"reference":[{"key":"2025082723051849000_18.4.e264200.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2022.05.065"},{"key":"2025082723051849000_18.4.e264200.2","doi-asserted-by":"crossref","DOI":"10.1186\/s12881-014-0091-8","article-title":"Further delineation of Loeys-Dietz syndrome type 4 in a family with mild vascular involvement and a TGFB2 splicing mutation","volume":"15","author":"Ritelli","year":"2014","journal-title":"BMC Med Genet"},{"key":"2025082723051849000_18.4.e264200.3","doi-asserted-by":"crossref","DOI":"10.3389\/fgene.2020.00479","article-title":"Identification of a Pathogenic TGFBR2 Variant in a Patient With Loeys-Dietz Syndrome","volume":"11","author":"Luo","year":"2020","journal-title":"Front Genet"},{"key":"2025082723051849000_18.4.e264200.4","doi-asserted-by":"crossref","first-page":"e590","DOI":"10.1097\/BPO.0000000000002131","article-title":"Tendon Healing in a Mouse Model of Loeys-Dietz Syndrome: Controlled Study Using a Patellar Tendon Transection Model","volume":"42","author":"Cottrill","year":"2022","journal-title":"J Pediatr Orthop"},{"key":"2025082723051849000_18.4.e264200.5","unstructured":"Miklovic T , Sieg VC . Ehlers-Danlos Syndrome . Nih.gov. StatPearls Publishing, 2023. Available: https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK549814\/"},{"key":"2025082723051849000_18.4.e264200.6","unstructured":"Malfait F , Wenstrup R . Classic ehlers-danlos syndrome, seattle. University of Washington; 2018. Available: https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK1244\/#:~:text=Clinical%20Description-,Classic%20Ehlers%2DDanlos%20syndrome%20(cEDS)%20is%20a%20connective%20tissue,healing%2C%20and%20generalized%20joint%20hypermobility"},{"key":"2025082723051849000_18.4.e264200.7","unstructured":"Fontana P , Genesio R , Casertano A , et al . Loeys\u2013Dietz syndrome type 4, caused by chromothripsis, involving the TGFB2 gene, Available: https:\/\/www.sciencedirect.com\/science\/article\/pii\/S0378111914000389#:~:text=Large%20deletions%20and%20non%2Dsense,%E2%80%93Dietz%20syndrome%2C%20type%204"},{"key":"2025082723051849000_18.4.e264200.8","doi-asserted-by":"publisher","DOI":"10.1111\/j.1524-475X.2008.00410.x"},{"key":"2025082723051849000_18.4.e264200.9","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1111\/iwj.13497","article-title":"The impact of incisional negative pressure wound therapy on the wound healing process after midline sternotomy","volume":"18","author":"Rashed","year":"2021","journal-title":"Int Wound J"},{"key":"2025082723051849000_18.4.e264200.10","article-title":"Negative pressure wound therapy for surgical wounds healing by primary closure","volume":"4","author":"Norman","year":"2022","journal-title":"Cochrane Database Syst Rev"},{"key":"2025082723051849000_18.4.e264200.11","doi-asserted-by":"crossref","first-page":"3081","DOI":"10.1111\/iwj.14183","article-title":"The effect of incisional negative pressure wound therapy on the improvement of postoperative cosmetic suture wounds and scar hyperplasia","volume":"20","author":"Liu","year":"2023","journal-title":"Int Wound J"},{"key":"2025082723051849000_18.4.e264200.12","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1111\/wrr.12858","article-title":"A systematic review evaluating the influence of incisional Negative Pressure Wound Therapy on scarring","volume":"29","author":"Zwanenburg","year":"2021","journal-title":"Wound Repair Regen"},{"key":"2025082723051849000_18.4.e264200.13","doi-asserted-by":"crossref","DOI":"10.1186\/s40792-020-01051-0","article-title":"Ehlers-Danlos syndrome type IV: a case report of a rare cause of spontaneous sigmoid perforation and enteroatmospheric fistulae in a child","volume":"6","author":"Kodikara","year":"2020","journal-title":"Surg Case Rep"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2024-264200","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,28]],"date-time":"2025-08-28T06:05:28Z","timestamp":1756361128000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2024-264200"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,4]]},"references-count":13,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2025,4,22]]},"published-print":{"date-parts":[[2025,4]]}},"alternative-id":["10.1136\/bcr-2024-264200"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2024-264200","relation":{},"ISSN":["1757-790X"],"issn-type":[{"type":"electronic","value":"1757-790X"}],"subject":[],"published":{"date-parts":[[2025,4]]}}}